Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000640454 | SCV000762045 | pathogenic | Telangiectasia, hereditary hemorrhagic, type 2 | 2018-04-08 | criteria provided, single submitter | clinical testing | This variant is an out-of-frame deletion of the genomic region encompassing the last 34 nucleotides of exon 4 and  exons 5-9 of the ACVRL1 gene (c.492-c.1378-239). This is expected to create a premature translational stop signal and result in an absent or disrupted protein product. This variant has not been reported in the literature in individuals with ACVRL1-related disease. Loss-of-function variants in ACVRL1 are known to be pathogenic (PMID: 15879500). For these reasons, this variant has been classified as Pathogenic. |